Rationale and design of the Pan African Pulmonary hypertension Cohort (PAPUCO) study: implementing a contemporary registry on pulmonary hypertension in Africa

Abstract

IntroductionPulmonary hypertension (PH) is a devastating, progressive disease with increasingly debilitating symptoms and usually shortened overall life expectancy due to a narrowing of the pulmonary vasculature and consecutive right heart failure. Little is known about PH in Africa, but limited reports suggest that PH is more prevalent in Africa compared with developed countries due to the high prevalence of risk factors in the region.Methods and analysisA multinational multicentre registry-type cohort study was established and tailored to resource-constraint settings to describe disease presentation, disease severity and aetiologies of PH, comorbidities, diagnostic and therapeutic management, and the natural course of PH in Africa. PH will be diagnosed by specialist cardiologists using echocardiography (right ventricular systolic pressure >35 mm Hg, absence of pulmonary stenosis and acute right heart failure), usually accompanied by shortness of breath, fatigue, peripheral oedema and other cardiovascular symptoms, ECG and chest X-ray changes in keeping with PH as per guidelines (European Society of Cardiology and European Respiratory Society (ESC/ERS) guidelines). Additional investigations such as a CT scan, a ventilation/perfusion scan or right heart catheterisation will be performed at the discretion of the treating physician. Functional tests include a 6 min walk test and the Karnofsky Performance Score. The WHO classification system for PH will be applied to describe the different aetiologies of PH. Several substudies have been implemented within the registry to investigate specific types of PH and their outcome at up to 24 months. Data will be analysed by an independent institution following a data analyse plan.Ethics and disseminationAll local ethics committees of the participating centres approved the protocol. The data will be disseminated through peer-reviewed journals at national and international conferences and public events at local care providers.

Gender disparities in pulmonary hypertension at a tertiary centre in Cameroon

“…First, echocardiography was the main diagnostic tool used for the diagnosis of PH, as opposed to gold-standard RHC. This limitation of our registry has been discussed previously [14] and has to do with the challenges of diagnosing PH in the resource-limited settings that are so common in SSA. Echocardiography is much more affordable and available, and poses almost no risk compared with the invasive procedure of RHC.…”

“…[13] The Pan African Pulmonary Hypertension Cohort (PAPUCO) study was established to investigate the epidemiology, causes and natural history of PH in Africa. [14,15] While the epidemiology of PH in Africa remains largely unknown, it is important to highlight the fact that clinical features of PH are often subtle and nonspecific, especially in the early stages, and may mimic other diseases, so that PH remains undiagnosed. Risk factors, epidemiology, clinical characteristics and outcomes of HF have gender variations that have been described among Caucasians.…”

“…The PAPUCO study design has been described in great detail, [14] and was registered on ClinicalTrials.gov (NCT02265887). In short, PAPUCO was a prospective pan-African multicentre cohort study of patients newly diagnosed with PH involving 12 specialist centres in SSA including Cameroon.…”

“…Details of the echocardiographic assessment have been described previously. [14] Data collection and study procedures…”

“…[14] General and clinical characteristics included age, sex and risk factors for cardiovascular disease (CVD). A family history of CVD was defined as a documented family history of CVD; hypertension as a systolic (diastolic) blood pressure of ≥140 (90) mmHg, a documented history of hypertension or prescribed antihypertensive medication; presence of diabetes as a fasting blood glucose level of ≥7 mmol/L, a documented history of diabetes or prescribed blood sugar-lowering agents; smoking as never having smoked, being an ex-smoker (if the patient had smoked for at least 3 years in the past, but had stopped at time of presentation) or being a current smoker (patients who smoked at least one cigarette per day at the time of presentation); and alcohol abuse as intake of either more than three (two for women) standard glasses of wine per day or more than ten (five for women) local beers (one local beer contains 28 g of alcohol) per week.…”